You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.

Jump To Section:

Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References XML

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2022-09-22 18:34:13 UTC

HMDB ID

HMDB0000522

Secondary Accession Numbers

HMDB00522

Metabolite Identification

Common Name

3-Methylglutaconic acid

Description

3-Methylglutaconic acid is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism (PMID: 7603789 ). 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. When present in sufficiently high levels, 3-methylglutaconic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-methylglutaconic acid are associated with at least five inborn errors of metabolism including 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methylglutaconic aciduria type I, 3-methylglutaconic aciduria type III, 3-methylglutaconic aciduria type IV, and guanidinoacetate methyltransferase deficiency (GAMT deficiency). 3-Methylglutaconic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

View in JSmol View Stereo Labels

Synonyms

Value	Source
3-Methylglutaconate	Generator
b-Methylglutaconate	HMDB
b-Methylglutaconic acid	HMDB
beta-Methylglutaconate	HMDB
beta-Methylglutaconic acid	HMDB
Β-methylglutaconic acid	HMDB
3-Methyl-2-pentenedioic acid	HMDB
3E-Methylglutaconate	HMDB
3-Methylglutaconic acid	HMDB, MeSH

Chemical Formula

C₆H₈O₄

Average Molecular Weight

144.1253

Monoisotopic Molecular Weight

144.042258744

IUPAC Name

(2E)-3-methylpent-2-enedioic acid

Traditional Name

β-methylglutaconic acid

CAS Registry Number

5746-90-7

SMILES

C\C(CC(O)=O)=C/C(O)=O

InChI Identifier

InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+

InChI Key

WKRBKYFIJPGYQC-DUXPYHPUSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Methyl-branched fatty acids

Alternative Parents

Substituents

Methyl-branched fatty acid
Unsaturated fatty acid
Dicarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

dicarboxylic acid (CHEBI:37245 )
Dicarboxylic acids (LMFA01170068 )

Ontology

Physiological effect

Adverse health effect

Digestive system disorder

Gastrointestinal disorder

Upper GI Disorder

Eosinophilic esophagitis - Concentration is elevated compared to control group (PMID: 17334708 , PMID: 32966057 , PMID: 22623390 , +128

Source	Link
Pubmed	PMID: 17334708
Pubmed	PMID: 32966057
Pubmed	PMID: 22623390
Pubmed	PMID: 16870009
Pubmed	PMID: 26910390
Pubmed	PMID: 8087979
Pubmed	PMID: 25105552
Pubmed	PMID: 17349089
Pubmed	PMID: 24023812
Pubmed	PMID: 12376931
Pubmed	PMID: 15992788
Pubmed	PMID: 24341803
Pubmed	PMID: 19468821
Pubmed	PMID: 9625050
Pubmed	PMID: 2420598
Pubmed	PMID: 27012787
Pubmed	PMID: 30830347
Pubmed	PMID: 19551947
Pubmed	PMID: 19309105
Pubmed	PMID: 2026685
Pubmed	PMID: 5727921
Pubmed	PMID: 22024767
Pubmed	PMID: 17031479
Pubmed	PMID: 9439441
Pubmed	PMID: 9625050
Pubmed	PMID: 28649521
Pubmed	PMID: 28649521
Pubmed	PMID: 12097436
Pubmed	PMID: 8257730
Pubmed	PMID: 3168222
Pubmed	PMID: 9869364
Pubmed	PMID: 3436096
Pubmed	PMID: 22157537
Pubmed	PMID: 1246461
Pubmed	PMID: 25691415
Pubmed	PMID: 18004736
Pubmed	PMID: 31506554
Pubmed	PMID: 20423563
Pubmed	PMID: 25536744
Pubmed	PMID: 21388201
Pubmed	PMID: 4903899
Pubmed	PMID: 14708889
Pubmed	PMID: 14967154
Pubmed	PMID: 6774165
Pubmed	PMID: 21389975
Pubmed	PMID: 3709568
Pubmed	PMID: 8311084
Pubmed	PMID: 2242313
Pubmed	PMID: 20549362
Pubmed	PMID: 12555941
Pubmed	PMID: 10731506
Pubmed	PMID: 28157703
Pubmed	PMID: 20576202
Pubmed	PMID: 12368406
Pubmed	PMID: 26066683
Pubmed	PMID: 16736096
Pubmed	PMID: 25597510
Pubmed	PMID: 22800120
Pubmed	PMID: 16139832
Pubmed	PMID: 17287286
Pubmed	PMID: 17301081
Pubmed	PMID: 30817767
Pubmed	PMID: 25851806
Pubmed	PMID: 2587127
Pubmed	PMID: 11893004
Pubmed	PMID: 10102904
Pubmed	PMID: 23430553
Pubmed	PMID: 12705346
Pubmed	PMID: 19075476
Pubmed	PMID: 3571488
Pubmed	PMID: 7776094
Pubmed	PMID: 31963255
Pubmed	PMID: 23093139
Pubmed	PMID: 22284503
Pubmed	PMID: 8503438
Pubmed	PMID: 1246461
Pubmed	PMID: 20502474
Pubmed	PMID: 15558695
Pubmed	PMID: 422265
Pubmed	PMID: 22308371
Pubmed	PMID: 20549362
Pubmed	PMID: 9177981
Pubmed	PMID: 16828325
Pubmed	PMID: 16460720
Pubmed	PMID: 28157703
Pubmed	PMID: 8487492
Pubmed	PMID: 23890588
Pubmed	PMID: 14586528
Pubmed	PMID: 12927681
Pubmed	PMID: 9686366
Pubmed	PMID: 9029640
Pubmed	PMID: 22148915
Pubmed	PMID: 27123458
Pubmed	PMID: 20971021
Pubmed	PMID: 3169394
Pubmed	PMID: 21670092
Pubmed	PMID: 23363473
Pubmed	PMID: 15519880
Pubmed	PMID: 2380628
Pubmed	PMID: 6616873
Pubmed	PMID: 1870421
Pubmed	PMID: 19754154
Pubmed	PMID: 9857238
Pubmed	PMID: 12771321
Pubmed	PMID: 2702744
Pubmed	PMID: 9554495
Pubmed	PMID: 19033659
Pubmed	PMID: 19033659
Pubmed	PMID: 22887155
Pubmed	PMID: 17474139
Pubmed	PMID: 18953024
Pubmed	PMID: 6774165
Pubmed	PMID: 11052553
Pubmed	PMID: 16939360
Pubmed	PMID: 22827565
Pubmed	PMID: 21736852
Pubmed	PMID: 12661026
Pubmed	PMID: 18818040
Pubmed	PMID: 23770102
Pubmed	PMID: 15651030
Pubmed	PMID: 11307586
Pubmed	PMID: 21389975
Pubmed	PMID: 1640293
Pubmed	PMID: 25712379
Pubmed	PMID: 23770102
Pubmed	PMID: 24390407
Pubmed	PMID: 26709303
Pubmed	PMID: 3354621
Pubmed	PMID: 29030856
Pubmed	PMID: 12706375
Pubmed	PMID: 9869358

)

Disposition

Biological location

Cellular substructure

Excreta

Feces
Urine

Organ

Placenta

Horse

Lagomorphs

Ovis

Sheep (Mutton, Lamb)

Caprae

Domestic goat

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	8.69 g/L	ALOGPS
logP	0.37	ALOGPS
logP	0.29	ChemAxon
logS	-1.2	ALOGPS
pKa (Strongest Acidic)	3.85	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	74.6 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	33.51 m³·mol⁻¹	ChemAxon
Polarizability	13.27 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	132.713	31661259
DarkChem	[M-H]-	126.531	31661259
AllCCS	[M+H]+	132.692	32859911
AllCCS	[M-H]-	127.887	32859911
DeepCCS	[M+H]+	132.032	30932474
DeepCCS	[M-H]-	128.199	30932474
DeepCCS	[M-2H]-	165.148	30932474
DeepCCS	[M+Na]+	140.591	30932474
AllCCS	[M+H]+	132.7	32859911
AllCCS	[M+H-H2O]+	128.6	32859911
AllCCS	[M+NH4]+	136.5	32859911
AllCCS	[M+Na]+	137.6	32859911
AllCCS	[M-H]-	127.9	32859911
AllCCS	[M+Na-2H]-	130.1	32859911
AllCCS	[M+HCOO]-	132.6	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
3-Methylglutaconic acid	C\C(CC(O)=O)=C/C(O)=O	2220.8	Standard polar	33892256
3-Methylglutaconic acid	C\C(CC(O)=O)=C/C(O)=O	1226.4	Standard non polar	33892256
3-Methylglutaconic acid	C\C(CC(O)=O)=C/C(O)=O	1383.3	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
3-Methylglutaconic acid,1TMS,isomer #1	C/C(=C\C(=O)O)CC(=O)O[Si](C)(C)C	1426.9	Semi standard non polar	33892256
3-Methylglutaconic acid,1TMS,isomer #2	C/C(=C\C(=O)O[Si](C)(C)C)CC(=O)O	1393.8	Semi standard non polar	33892256
3-Methylglutaconic acid,2TMS,isomer #1	C/C(=C\C(=O)O[Si](C)(C)C)CC(=O)O[Si](C)(C)C	1470.6	Semi standard non polar	33892256
3-Methylglutaconic acid,1TBDMS,isomer #1	C/C(=C\C(=O)O)CC(=O)O[Si](C)(C)C(C)(C)C	1661.6	Semi standard non polar	33892256
3-Methylglutaconic acid,1TBDMS,isomer #2	C/C(=C\C(=O)O[Si](C)(C)C(C)(C)C)CC(=O)O	1637.9	Semi standard non polar	33892256
3-Methylglutaconic acid,2TBDMS,isomer #1	C/C(=C\C(=O)O[Si](C)(C)C(C)(C)C)CC(=O)O[Si](C)(C)C(C)(C)C	1894.3	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-0015-9200000000-ff306cede85d252e0d4c	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (2 TMS) - 70eV, Positive	splash10-00di-9340000000-0b493822a103307b8f66	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutaconic acid GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Negative-QTOF	splash10-000f-9000000000-fa6952af79fc3b2759fe	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Negative-QTOF	splash10-0a4j-9000000000-3a5a017393a0592a65ff	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Negative-QTOF	splash10-000f-9000000000-14ef6163925508d3f666	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Negative-QTOF	splash10-0002-9000000000-ada755597943f74819c4	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Negative-QTOF	splash10-0002-9000000000-70f10348ac9e84418765	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Negative-QTOF	splash10-0a4i-9000000000-5a9ee69bdb3960321fa6	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Positive-QTOF	splash10-002b-2900000000-b6862aeffb1aa9fae1d0	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Positive-QTOF	splash10-000i-9300000000-7ab01dfb0b86abdac817	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Positive-QTOF	splash10-0006-9000000000-3692ceff17a888ae8836	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Negative-QTOF	splash10-0006-5900000000-dc4d4a80a164f3fd1fea	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Negative-QTOF	splash10-0007-8900000000-7ece848c8011e4a20200	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Negative-QTOF	splash10-0a7l-9300000000-8b54483c15a17429b9f8	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Negative-QTOF	splash10-0002-9200000000-70569e6181007374644c	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Negative-QTOF	splash10-000t-9000000000-9426c88487da5c337c1b	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Negative-QTOF	splash10-000x-9000000000-60467181d27d878d6aca	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 10V, Positive-QTOF	splash10-0002-9400000000-79724b8b1198fb442949	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 20V, Positive-QTOF	splash10-001j-9000000000-4f399595435fe16b3880	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutaconic acid 40V, Positive-QTOF	splash10-001c-9000000000-1ee161ce72a30f1b0814	2021-09-24	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, H₂O, predicted)	2022-08-18	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)	2022-02-11	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI-, Adduct: [M-H]-)	2022-02-11	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Urine

Tissue Locations

Placenta

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Cerebrospinal Fluid (CSF)	Detected and Quantified	<10 uM	Adult (>18 years old)	Female	Normal	16541463	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Urine	Detected and Quantified	<6 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	16736096	details
Urine	Detected and Quantified	0-26 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	<10 umol/mmol creatinine	Not Specified	Not Specified	Normal	10484795	details
Urine	Detected and Quantified	<20 umol/mmol creatinine	Not Specified	Not Specified	Normal	19015156	details
Urine	Detected and Quantified	<1500 umol/mmol creatinine	Not Specified	Not Specified	Normal	22797137	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	<25 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	15505778	details
Urine	Detected and Quantified	1.0 - 6.5 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	16541463	details
Urine	Detected and Quantified	0-0.2 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	0-4 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	0-6.0 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	1.8-7.6 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	<6 umol/mmol creatinine	Not Specified	Not Specified	Normal	23705938	details
Urine	Detected and Quantified	<12.42 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	<25 umol/mmol creatinine	Not Specified	Not Specified	Normal	15505778	details
Urine	Detected and Quantified	<12 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	25597510	details
Urine	Detected and Quantified	<20 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	25597510	details
Urine	Detected and Quantified	<0.0200 umol/mmol creatinine	Not Specified	Not Specified	Normal	16527507	details
Urine	Detected and Quantified	6.2 (2.8-8.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	10.707 +/- 4.243 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	7.5 (3.4-9.5) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	15.86 +/- 6.052 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Expected but not Quantified	Not Quantified	Not Specified	Not Specified	Cancer patients undergoing total body irradiation	31424920	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	125 uM	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	16541463	details
Urine	Detected and Quantified	20-488 umol/mmol creatinine	Children (1-13 years old)	Both	Sengers syndrome	16736096	details
Urine	Detected and Quantified	750.0 (500.0-1000.0) umol/mmol creatinine	Children (1-13 years old)	Both	3-Methylglutaconic Aciduria (TYPE I)	MetaGene: Metabol...	details
Urine	Detected and Quantified	0-230 umol/mmol creatinine	Infant (0-1 year old)	Male	3-methylglutaconic aciduria type II, X-linked	10484795	details
Urine	Detected and Quantified	16-68 umol/mmol creatinine	Adolescent (13-18 years old)	Female	3-Methylglutaconic Aciduria (Type IV)	19015156	details
Urine	Detected and Quantified	4-200 umol/mmol creatinine	Children (1-13 years old)	Both	3-Methylglutaconic Aciduria (Type IV)	19015156	details
Urine	Detected and Quantified	13000 umol/mmol creatinine	Children (1-13 years old)	Male	3-Methylglutaconic Aciduria (Type V)	22797137	details
Urine	Detected and Quantified	3714 umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	15505778	details
Urine	Detected and Quantified	32-51 umol/mmol creatinine	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	16541463	details
Urine	Detected and Quantified	62-90 umol/mmol creatinine	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	16541463	details
Urine	Detected and Quantified	4166.4 umol/mmol creatinine	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	23705938	details
Urine	Detected and Quantified	3714 umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	15505778	details
Urine	Detected and Quantified	29-109 umol/mmol creatinine	Children (1-13 years old)	Female	3-Methylglutaconic Aciduria (Type VII)	25597510	details
Urine	Detected and Quantified	34-160 umol/mmol creatinine	Adolescent (13-18 years old)	Female	3-Methylglutaconic Aciduria (Type VII)	25597510	details
Urine	Detected and Quantified	52-93 umol/mmol creatinine	Infant (0-1 year old)	Female	3-Methylglutaconic Aciduria (Type VII)	25597510	details
Urine	Detected and Quantified	0.0160-0.196 umol/mmol creatinine	Children (1-13 years old)	Female	3-Methylglutaconic Aciduria (Type VI)	16527507	details
Urine	Detected and Quantified	94-141 umol/mmol creatinine	Adult (>18 years old)	Not Specified	3-Methylglutaconic Aciduria (TYPE I)	20855850	details
Urine	Detected and Quantified	570 umol/mmol creatinine	Children (1-13 years old)	Not Specified	3-Methylglutaconic Aciduria (TYPE I)	20855850	details
Urine	Detected and Quantified	10.714 +/- 4.929 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	12.053 +/- 6.469 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details
Urine	Detected and Quantified	14.626 +/- 7.998 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	4.5 (0.00-9.0) umol/mmol creatinine	Adult (>18 years old)	Both	3-Hydroxy-3-methylglutaryl-CoA lyase deficiency	MetaGene: Metabol...	details
Urine	Detected and Quantified	17.038 +/- 8.547 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details
Urine	Detected and Quantified	5070.0 (140.0-10000.0) umol/mmol creatinine	Children (1-13 years old)	Both	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	MetaGene: Metabol...	details

Associated Disorders and Diseases

Disease References

3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Bischof F, Nagele T, Wanders RJ, Trefz FK, Melms A: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in an adult with leukoencephalopathy. Ann Neurol. 2004 Nov;56(5):727-30. [PubMed:15505778 ] Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
3-Methylglutaconic aciduria type I
Engelke UF, Kremer B, Kluijtmans LA, van der Graaf M, Morava E, Loupatty FJ, Wanders RJ, Moskau D, Loss S, van den Bergh E, Wevers RA: NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR Biomed. 2006 Apr;19(2):271-8. [PubMed:16541463 ] Wortmann SB, Kremer BH, Graham A, Willemsen MA, Loupatty FJ, Hogg SL, Engelke UF, Kluijtmans LA, Wanders RJ, Illsinger S, Wilcken B, Cruysberg JR, Das AM, Morava E, Wevers RA: 3-Methylglutaconic aciduria type I redefined: a syndrome with late-onset leukoencephalopathy. Neurology. 2010 Sep 21;75(12):1079-83. doi: 10.1212/WNL.0b013e3181f39a8a. [PubMed:20855850 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
3-methylglutaconic aciduria type II, X-linked
Cantlay AM, Shokrollahi K, Allen JT, Lunt PW, Newbury-Ecob RA, Steward CG: Genetic analysis of the G4.5 gene in families with suspected Barth syndrome. J Pediatr. 1999 Sep;135(3):311-5. [PubMed:10484795 ]
3-Methylglutaconic Aciduria type VI
Wortmann S, Rodenburg RJ, Huizing M, Loupatty FJ, de Koning T, Kluijtmans LA, Engelke U, Wevers R, Smeitink JA, Morava E: Association of 3-methylglutaconic aciduria with sensori-neural deafness, encephalopathy, and Leigh-like syndrome (MEGDEL association) in four patients with a disorder of the oxidative phosphorylation. Mol Genet Metab. 2006 May;88(1):47-52. doi: 10.1016/j.ymgme.2006.01.013. Epub 2006 Mar 9. [PubMed:16527507 ]
3-Methylglutaconic Aciduria type IV
Wortmann SB, Rodenburg RJ, Jonckheere A, de Vries MC, Huizing M, Heldt K, van den Heuvel LP, Wendel U, Kluijtmans LA, Engelke UF, Wevers RA, Smeitink JA, Morava E: Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy. Brain. 2009 Jan;132(Pt 1):136-46. doi: 10.1093/brain/awn296. Epub 2008 Nov 16. [PubMed:19015156 ]
3-Methylglutaconic Aciduria type V
Ojala T, Polinati P, Manninen T, Hiippala A, Rajantie J, Karikoski R, Suomalainen A, Tyni T: New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies. Pediatr Res. 2012 Oct;72(4):432-7. doi: 10.1038/pr.2012.92. Epub 2012 Jul 13. [PubMed:22797137 ]
3-Methylglutaconic aciduria type VII
Wortmann SB, Zietkiewicz S, Kousi M, Szklarczyk R, Haack TB, Gersting SW, Muntau AC, Rakovic A, Renkema GH, Rodenburg RJ, Strom TM, Meitinger T, Rubio-Gozalbo ME, Chrusciel E, Distelmaier F, Golzio C, Jansen JH, van Karnebeek C, Lillquist Y, Lucke T, Ounap K, Zordania R, Yaplito-Lee J, van Bokhoven H, Spelbrink JN, Vaz FM, Pras-Raves M, Ploski R, Pronicka E, Klein C, Willemsen MA, de Brouwer AP, Prokisch H, Katsanis N, Wevers RA: CLPB mutations cause 3-methylglutaconic aciduria, progressive brain atrophy, intellectual disability, congenital neutropenia, cataracts, movement disorder. Am J Hum Genet. 2015 Feb 5;96(2):245-57. doi: 10.1016/j.ajhg.2014.12.013. Epub 2015 Jan 15. [PubMed:25597510 ]
Sengers syndrome
Di Rosa G, Deodato F, Loupatty FJ, Rizzo C, Carrozzo R, Santorelli FM, Boenzi S, D'Amico A, Tozzi G, Bertini E, Maiorana A, Wanders RJ, Dionisi-Vici C: Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria. J Inherit Metab Dis. 2006 Aug;29(4):546-50. doi: 10.1007/s10545-006-0279-y. Epub 2006 May 30. [PubMed:16736096 ]

Associated OMIM IDs

246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
250950 (3-Methylglutaconic aciduria type I)
610247 (Eosinophilic esophagitis)
302060 (3-methylglutaconic aciduria type II, X-linked)
614739 (3-Methylglutaconic Aciduria type VI)
250951 (3-Methylglutaconic Aciduria type IV)
610198 (3-Methylglutaconic Aciduria type V)
616271 (3-Methylglutaconic aciduria type VII)
212350 (Sengers syndrome)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022090

KNApSAcK ID

C00052150

Chemspider ID

1267861

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

3-Methylglutaconic acid

METLIN ID

5507

PubChem Compound

1551553

PDB ID

Not Available

ChEBI ID

37245

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000182

Good Scents ID

Not Available

References

Synthesis Reference

Aoyama, Takayuki; Kato, Kazuo; Shimada, Nobuyoshi; Fujii, Akio; Takita, Tomohisa. Production of 3-methylglutaconic acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp.

Material Safety Data Sheet (MSDS)

Not Available

General References

Mitchell GA, Jakobs C, Gibson KM, Robert MF, Burlina A, Dionisi-Vici C, Dallaire L: Molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase deficiency. Prenat Diagn. 1995 Aug;15(8):725-9. [PubMed:7479590 ]
Holme E, Greter J, Jacobson CE, Larsson NG, Lindstedt S, Nilsson KO, Oldfors A, Tulinius M: Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria. Pediatr Res. 1992 Dec;32(6):731-5. [PubMed:1287564 ]
Costeff H, Elpeleg O, Apter N, Divry P, Gadoth N: 3-Methylglutaconic aciduria in "optic atrophy plus". Ann Neurol. 1993 Jan;33(1):103-4. [PubMed:8494328 ]
Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behr's syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. [PubMed:1384336 ]
Kelley RI: Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry. Clin Chim Acta. 1993 Nov 15;220(2):157-64. [PubMed:8111960 ]
Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed:7603789 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for 3-Methylglutaconic acid (HMDB0000522)

MOL for HMDB0000522 (3-Methylglutaconic acid)

3D MOL for HMDB0000522 (3-Methylglutaconic acid)

3D SDF for HMDB0000522 (3-Methylglutaconic acid)

3D-SDF for HMDB0000522 (3-Methylglutaconic acid)

PDB for HMDB0000522 (3-Methylglutaconic acid)

3D PDB for HMDB0000522 (3-Methylglutaconic acid)

SMILES for HMDB0000522 (3-Methylglutaconic acid)

INCHI for HMDB0000522 (3-Methylglutaconic acid)

Structure for HMDB0000522 (3-Methylglutaconic acid)

3D Structure for HMDB0000522 (3-Methylglutaconic acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra